title: Kawasaki disease associated with reactive hemophagocytic lymphohistiocytosis.
abstract: We report a case of Kawasaki disease with significant coronary artery aneurysms subsequently associated with reactive hemophagocytic lymphohistiocytosis in a young child with low T-cell perforin expression and NK-cell dysfunction. The patient was treated with a selective T-cell costimulation modulator in an effort to regulate T-cells. This case is unique for several reasons: (1) the severe degree of coronary artery aneurysms; (2) low T-cell perforin and NK-cell values; and (3) treatment with a selective T-cell costimulation modulator, none of which has been described in prior cases.
authors.affiliation: Department of Pediatrics, Yale-New Haven Children's Hospital, 20 York Street, New Haven, CT 06511, USA. Christy.Cummings@yale.edu
authors.forename: Christy
authors.initials: C
authors.lastname: Cummings
authors.forename: Paul
authors.initials: P
authors.lastname: McCarthy
authors.forename: Jack
authors.initials: J
authors.lastname: van Hoff
authors.forename: George
authors.initials: G
authors.lastname: Porter
mesh_terms: D002648:Child; D003323:Coronary Aneurysm / Q000150:complications / Q000276:immunology / Q000378:metabolism; D006801:Humans; D007694:Killer Cells, Natural / Q000276:immunology / Q000378:metabolism*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000188:drug therapy / Q000276:immunology* / Q000378:metabolism; D008262:Macrophage Activation / Q000276:immunology; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications / Q000188:drug therapy / Q000276:immunology* / Q000378:metabolism; D054353:Perforin / Q000276:immunology / Q000378:metabolism*
chemical_list: D054353:Perforin